12.1 abnormality in metabolism

C

LINICAL

C

ORRELATION

Usman Sumo Friend Tambunan Arli Aditya Parikesit

Geraldine Marcella L

Bioinformatics Group Department of Chemistry

 Diabetes Mellitus

 Galactosemia

 Protein Malnutrition

 Reye’s Syndrome

P

ROTEIN

M

ALNUTRITION

Definition

 Malnutrition is the result of extended inadequate

intake or severe illness on body composition and function and affect all system of the body.

Two types of malnutrition:

 Protein malnutrition, refers to kwashiorkor.

 Protein Calorie (Energy) Malnutrition, refers to

K

WASHIORKOR

 Usually occur in children aged

1-4 years although can be occurred in older children or adults.

 Symptoms: swollen abdomen

(known as pot belly), edema,

failure to gain weight and failure of linear growth, hair changes, depigmented skin.

 Long term impact is on the

physical and mental

P

ROTEIN

C

ORRELATION

 Protein is composed from

amino acids.

 Amino acid can be classified

into two types, which are: essential amino acid and nonessential amino acid.

 Essential amino acid cannot be

produced in human body, it supplied from food we eat.

 Kwashiorkor can be occurred

when there is no food supply to body or the food doesn’t

 Essential amino acids are

very important because they are precursor for many

others metabolism pathways.

 For example: Phenylalanine

 In humans, melanin is the

primary determinant of human skin color and also found in hair

Protein source

Limiting amino acid

Wheat Lysine

Rice lysine

Legumes tryptophan

Maize lysine and tryptophan

P

OSSIBLE

C

AUSES OF THE

D

EVELOPMENT OF

K

WASHIORKOR

 The protein deficiency, in combination with

energy and micronutrient deficiency, is not the key factor of the development of kwashiorkor.

 One important factor in development of

kwashiorkor are aflatoxin poisoning.

 Aflatoxin can cause mycotoxin. Aflatoxins are

naturally produced by aspergillus, a fungus.

 After aflatoxin enter the body, it will be

metabolized in liver with enzyme cyttochrome p450 help produce reactive intermediate

aflatoxin M1, the final product is epoxide.

The correlation between damage liver

DNA and development of kwashiorkor

 Protein catabolism involves

urea cycle that take place in liver. The product is urea or uric acid and will be

removed from body.

 Because of the liver is

damage and cannot do the urea cycle, it cannot

converted amonia compound to urea and cause the

accumulation of amonia, resulting the failure

damage.

 Liver is the place to

produced many serum

proteins. With the damage of liver, symptoms of

T

REATMENT OF

K

WASHIORKOR

 Treatment of kwashiorkor begins with

rehydration. Subsequent increase in food intake must proceed slowly, beginning with

Reye’s Syndrome

 Reye’s syndrome is a potentially fatal disease

that affect all organs of body, especially liver and brain.

 In almost case, reye’s syndrome is associated

with viral infection. Such as: influenza, cold, or chicken pox.

 The mechanism of how Reye’s syndrome occurs is

S

YMPTOMS AND

S

IGN

Reye’s syndrome progress

through five stages, which are:

Stage I

 heavy vomiting  Confusion

 Nightmares

Stage II

 Fatty liver (found by biopsy)  Hyperventilation

 Hyperactive reflexes

Stage III

 Possible coma

 Possible cerebral edema

 Rarely, respiratory arrest

Stage IV

 Deepening coma

 Large pupils with minimal

response to light

 Minimal but still present hepatic

dysfunction

Stage V

 Very rapid onset following stage

IV

 Deep coma  Seizures

 Respiratory failure  Flaccidity

 Extremely high blood ammonia

(above 300mg per 100mL of blood)

C

AUSES

 Reye’s syndrome causes fatty accumulation in the

organs of the body, especially in liver.

 In the brain, it causes accumulation of fluid

(edema), and rising the pressure.

 This pressure squeezes blood vessels, preventing

blood from entering the brain.

 Untreated, this pressure increase leads to brain

T

EST

The following tests may be used to diagnose Reye syndrome:

 Blood chemistry tests show low blood sugar

(glucose) levels

 Liver function tests show higher than normal

levels of liver enzymes

 Serum ammonia test may be higher than normal

 Liver biopsy

 Spinal tap

 Head CT or head MRI scan help rule out other

C

ONCLUSION

 Kwashiorkor is one of the more severe forms of

protein malnutrition and is caused by inadequate protein intake.

 The development of kwashiorkor can lead to

damage liver DNA.

 Reye’s syndrome is a fatal disease that can

damage organs of body, especially liver and brain

 The mechanism of reye’s syndrome remains

R

EFERENCES

http://en.wikipedia.org/wiki/Kwashiorkor

http://www.healthscout.com/ency/1/ImagePages/9563.html http://en.wikipedia.org/wiki/Aflatoxin

http://www.unisanet.unisa.edu.au/08366/h&p2ptn.htm#intro

http://en.wikipedia.org/wiki/Urea_cycle

http://healthguide.howstuffworks.com/reye-syndrome-dictionary.htm http://www.reyessyndrome.org/what.html

http://en.wikipedia.org/wiki/Reye's_syndrome